Romano-Ward syndrome

ロマノ・ワード症候群 RWS

WordNet

1. relating to or characteristic of people of Rome; "Roman virtues"; "his Roman bearing in adversity"; "a Roman nose"
2. a resident of modern Rome
3. an inhabitant of the ancient Roman Empire
4. of or relating to or supporting Romanism; "the Roman Catholic Church" (同)R.C., Romanist, romish, Roman_Catholic, popish, papist, papistic, papistical
5. of or relating to or derived from Rome (especially ancient Rome); "Roman architecture"; "the old Roman wall" (同)Romanic
6. characteristic of the modern type that most directly represents the type used in ancient Roman inscriptions
7. block forming a division of a hospital (or a suite of rooms) shared by patients who need a similar kind of care; "they put her in a 4-bed ward" (同)hospital ward
8. a district into which a city or town is divided for the purpose of administration and elections
9. a person who is under the protection or in the custody of another
10. English economist and conservationist (1914-1981) (同)Barbara Ward, Baroness Jackson of Lodsworth
11. United States businessman who in 1872 established a successful mail-order business (1843-1913) (同)Montgomery Ward, Aaron Montgomery Ward
12. English writer of novels who was an active opponent of the womens suffrage movement (1851-1920) (同)Mrs. Humphrey Ward, Mary Augusta Arnold Ward
13. a pattern of symptoms indicative of some disease
14. a complex of concurrent things; "every word has a syndrome of meanings"

PrepTutorEJDIC

1. (古代・現代の)『ローマの』,ローマ人の,ローマ文化の / ローマカトリック教会(教徒)の / (特に古代の)『ローマ人』,ローマ市民 / (現代の)ローマ人
2. 〈C〉『病棟』,病室 / 〈C〉(刑務所の)監房 / 〈U〉(未成年者などの)後見,保護;〈C〉被後見人 / 〈C〉(行政区画の)『区』 / 〈U〉警備,見張り / 〈危険・病気など〉‘を'避ける,‘から'身を守る《+off+名,+名+off》
3. (疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/10/07 09:43:06」(JST)

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Romano-Ward syndrome
Classification and external resources
Schematic representation of normal ECG trace (sinus rhythm), with waves, segments, and intervals labeled.
ICD-9	794.31
OMIM	192500
DiseasesDB	11661
MeSH	D029597

Romano-Ward syndrome, is the major variant of long QT syndrome. It is a condition that causes a disruption of the heart's normal rhythm. This disorder is a form of long QT syndrome, which is a heart condition that causes the cardiac muscle to take longer than usual to recharge between beats. If untreated, the irregular heartbeats can lead to fainting, seizures, or sudden death.

Inheritance

Romano-Ward syndrome is inherited in an autosomal dominant pattern.

Romano-Ward syndrome is inherited in an autosomal dominant pattern. It is the most common form of inherited long QT syndrome, affecting an estimated 1 in 5,000 people worldwide, although more people may be affected but never experience any signs or symptoms of the condition.

Causes

Mutations in the ANK2, KCNE1, KCNE2, KCNH2, KCNQ1, and SCN5A genes cause Romano-Ward syndrome. The proteins made by most of these genes form channels that transport positively-charged ions, such as potassium and sodium, in and out of cells. In cardiac muscle, these ion channels play critical roles in maintaining the heart's normal rhythm. Mutations in any of these genes alter the structure or function of channels, which changes the flow of ions between cells. A disruption in ion transport alters the way the heart beats, leading to the abnormal heart rhythm characteristic of Romano-Ward syndrome.

Unlike most genes related to Romano-Ward syndrome, the ANK2 gene does not produce an ion channel. The protein made by the ANK2 gene ensures that other proteins, particularly ion channels, are inserted into the cell membrane appropriately. A mutation in the ANK2 gene likely alters the flow of ions between cells in the heart, which disrupts the heart's normal rhythm and results in the features of Romano-Ward syndrome.

This article incorporates public domain text from The U.S. National Library of Medicine

Treatment

An imbalance between the right and left sides of the sympathetic nervous system may play a role in the etiology of this syndrome. The imbalance can be temporarily abolished with a left stellate ganglion block, which shorten the QT interval. If this is successful, surgical ganglionectomy can be performed as a permanent treatment.

References

External links

• GeneReviews/NCBI/NIH/UW entry on Romano-Ward Syndrome
• synd/466 at Who Named It?

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. 先天性QT延長症候群の臨床的特徴 clinical features of congenital long qt syndrome
• 2. 先天性および後天性ＱＴ延長症候群の遺伝学 genetics of congenital and acquired long qt syndrome
• 3. 先天性ＱＴ延長症候群の診断 diagnosis of congenital long qt syndrome
• 4. 頻脈を有する小児に対するアプローチ approach to the child with tachycardia
• 5. 小児および思春期における失神の原因 causes of syncope in children and adolescents

English Journal

• A KCNE1 missense variant (V47I) causing exercise-induced long QT syndrome (Romano Ward).

• Ryan JJ, Kalscheur M, Dellefave L, McNally E, Archer SL.
• International journal of cardiology.Int J Cardiol.2012 Apr 19;156(2):e33-5. Epub 2011 Sep 9.
• PMID 21907427

• Dominant-negative control of cAMP-dependent IKs upregulation in human long-QT syndrome type 1.

• Heijman J, Spätjens RL, Seyen SR, Lentink V, Kuijpers HJ, Boulet IR, de Windt LJ, David M, Volders PG.SourceDepartment of Cardiology, Cardiovascular Research Institute Maastricht, Maastricht University Medical Centre, PO Box 5800, 6202 AZ Maastricht, The Netherlands.
• Circulation research.Circ Res.2012 Jan 20;110(2):211-9. Epub 2011 Nov 17.
• RATIONALE: The mutation A341V in the S6 transmembrane segment of KCNQ1, the α-subunit of the slowly activating delayed-rectifier K(+) (I(Ks)) channel, predisposes to a severe long-QT1 syndrome with sympathetic-triggered ventricular tachyarrhythmias and sudden cardiac death.OBJECTIVE: Several geneti
• PMID 22095730

Japanese Journal

• 水泳中に心室細動を発症した Romano-Ward 症候群の1症例

• 澤村 淳,菅野 正寛,久保田 信彦,上垣 慎二,早川 峰司,渡邉 昌也,丸藤 哲
• 日本救急医学会雑誌 22(5), 219-223, 2011-05-15
• NAID 10029368483

• 先天性QT延長症候群に伴う臨床像と心事故に関する検討 : サブタイプによる心事故の誘因と治療効果の違い

• 玉城 明実,志賀 剛,萩原 誠久,松岡 瑠美子,笠貫 宏
• 東京女子医科大学雑誌 77(12), 733-738, 2007-12
• … Data about the clinical course of Japanese congenital long-QT syndrome (LQTS) patients are limited. … 54 LQTS patients (1 Jervell and Lange-Nielsen, 53 Romano-Ward 53; …
• NAID 110007525336

Related Links

• Romano-Ward syndrome - Genetics Home Reference

Romano-Ward syndrome is a condition that causes a disruption of the heart's normal rhythm (arrhythmia). This disorder is a form of long QT syndrome, which is a heart condition that causes the heart (cardiac) muscle to ...

• Romano-Ward syndrome - definition of Romano-Ward ...

Romano-Ward syndrome [rō·mä′nō wôrd] Etymology: C. Romano, Italian physician, b. 1923; O.C. Ward, Irish physician, 20th century an autosomal-dominant form of the long QT syndrome, characterized by syncope and sometimes ...

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★リンクテーブル★

リンク元	「ロマノ・ワード症候群」「RWS」
関連記事	「ward」「syndrome」

「ロマノ・ワード症候群」

　　[★]

英

Romano-Ward syndrome, RWS

同

Romano-Ward症候群

関

先天性QT延長症候群

「RWS」

　　[★] ロマノ・ワード症候群, Romano-Ward syndrome

「ward」

　　[★]

• n.

• 病棟、(行政)区

　 　 　 　

「syndrome」

　　[★]

• n.

• 症候群